ISSN 1941-5923 © Am J Case Rep, 2017; 18: 894-901 DOI: 10.12659/AJCR.903702

Spindle Cell Oncocytoma of the Anterior Pituitary Presenting with an Acute Clinical Course Due To Intraventricular Hemorrhage. A Case Report and Review of Literature

Received: 2017.02.09 Accepted: 2017.04.30 Published: 2017.08.17

Authors’ ABCDEF Contribution: Mostafa Osman Study Design  A ACD Andrew Wild Data Collection  B Statistical Analysis  C Data Interpretation  D Manuscript Preparation  E Literature Search  F Funds Collection  G



Corresponding Author: Conflict of interest:

Department of Neurosurgery, Dr. Soliman Fakeeh Hospital, Jeddah, Saudi Arabia

Mostafa Osman, e-mail: [email protected] None declared

Patient: Male, 56 Final Diagnosis: Spindle cell oncocytoma of the adenohypophysis Symptoms: Disturbed conscious level • visual disturbances Medication: — Clinical Procedure: Urgent craniotomy Specialty: Neurosurgery

Objective: Background:



Case Report:



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Rare disease Spindle cell oncocytoma (SCO) is a rare nonfunctioning neoplasm of the adenohypophysis, and was first described in 2002. SCO has been categorized as a separate entity by the 2007 World Health Organization (WHO) and is classified as a Grade 1 tumor of the central nervous system (CNS). Review of the literature has shown that 33 cases of SCO have been reported to date, and most of them presented with a mass effect or with panhypopituitarism. However, all reported cases have described the tendency of SCO to be hypervascular on imaging and histology. We detail the first reported case of SCO to present with acute symptoms (pituitary apoplexy) and intraventricular hemorrhage, and review the literature on SCO. We report the case of 56-year-old man who presented suddenly with a severe headache and an altered level of consciousness. Brain magnetic resonance imaging (MRI) showed a suprasellar mass with hemorrhagic areas within the tumor and bleeding into the lateral ventricle with chiasmal and hypothalamic compression. The patient underwent urgent craniotomy, tumor resection and placement of an external ventricular drain (EVD). Histology and immunohistochemistry supported a diagnosis of SCO. SCO of the adenohypophysis should be considered in patients who present suddenly with symptoms of pituitary apoplexy and intraventricular hemorrhage which may worsen the prognosis. Brain Neoplasms • Pituitary Apoplexy • Pituitary Neoplasms https://www.amjcaserep.com/abstract/index/idArt/903702

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Osman M. et al.: Spindle cell oncocytoma of the adenohypophysis © Am J Case Rep, 2017; 18: 894-901

Background Primary tumors of the anterior pituitary, or adenohypophysis, account for between 10% and 15% of all intracranial tumors. The spectrum of tumors at this site includes the most common, the pituitary adenoma, followed by craniopharyngioma, meningioma, oncocytoma, pituitcytoma and granular cell tumor [1]. SCO of the adenohypophysis is a rare, benign, primary tumor in the sellar region, accounting for 0.1% 0.4% of all sellar tumors [2]. SCO was described as a new entity in 2002 by Roncaroli and colleagues and was classified as a distinct non-adenomatous sellar mass in the 2007 World Health Organization (WHO) classification [3,4]. Due to its rarity, little information is available regarding the imaging features and surgical characteristics of SCO. To our knowledge, there are only 34 cases reported to date (including our case). Most cases present with symptoms of slow onset that progress with as the tumor increases in size, resulting in compression of surrounding structures. Few cases presented with acute symptoms. Our case report is the first to describe a case presenting clinically with intratumoral bleeding and hemorrhage into the ventricles.

Case Report A 56-year-old man presented to the emergency room (ER) with acute onset of a severe headache, vomiting, neck pain, back pain, and reduced level of consciousness. His son reported recently impaired visual acuity. On examination, the patient was stable and the Glasgow Coma Scale (GCS) score was 13/15 with no other neurological deficit. Following initial management in ER, the patient was sent for further investigations. Magnetic resonance imaging (MRI) of the brain was performed using a 1.5 Tesla Siemens Avanto scanner. Multi-planar, multisequence images were obtained. Brain MRI showed a sellar and suprasellar macro-adenoma measuring 2.5×4.4×2.5 cm in its maximum anteroposterior, craniocaudal, and transverse dimensions, respectively, with intralesional bleeding, and intraventricular hemorrhage extending into the fourth ventricle. Mild ventricular system dilatation was also noted, with chiasmal and hypothalamic compression (Figures 1, 2). The patient went for urgent sub-frontal craniotomy for tumor resection and placement of an external ventricular drain (EVD). Intra-operative tumor hypervascularity gave the macroscopic appearance of meningioma, and profuse bleeding limited the surgical resection. A sample of cerebrospinal fluid (CSF) was sent for analysis, and the tumor specimen was sent for histopathological examination and microbiological culture and sensitivity.

There were no significant abnormalities found on analysis of the CSF, Gram stain or culture and sensitivity. Light microscopy (Figure 3) with hematoxylin and eosin (H&E) staining showed interlacing fascicles of spindle cells, and epithelioid cells with eosinophilic cytoplasm containing numerous mitochondria (oncocytic change). The absence of cellular atypia, mitoses, invasion, and necrosis, together with a low cell proliferation rate on immunohistochemical staining with antibodies to Ki67 all supported the benign nature of the tumor. Immunohistochemical staining also showed that the tumor cells were uniformly positive for vimentin, S100, epithelial membrane antigen (EMA), and thyroid transcription factor-1 (TTF-1), and focally positive for glial fibrillary acidic protein (GFAP), and was negative for immunostaining with antibodies to CD34, smooth muscle actin (SMA), desmin, and pan-cytokeratin (CK). Serological findings showed that the basal level of growth hormone (GH) was

Spindle Cell Oncocytoma of the Anterior Pituitary Presenting with an Acute Clinical Course Due To Intraventricular Hemorrhage. A Case Report and Review of Literature.

BACKGROUND Spindle cell oncocytoma (SCO) is a rare nonfunctioning neoplasm of the adenohypophysis, and was first described in 2002. SCO has been categ...
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